The role of postoperative radiotherapy in eccrine porocarcinoma: a multidisciplinary systematic review.

OBJECTIVE: Eccrine porocarcinoma (EPC) is a malignant adnexal tumor accounting for about 0.005% of skin tumors. The standard treatment of EPC is the complete surgical excision of the primary lesion and of the clinically involved lymph nodes. There is limited evidence regarding the role of radiotherapy (RT) in managing EPC after surgery. Therefore, the aim of this multidisciplinary systematic review is to analyze the available evidence about postoperative RT in the curative treatment of EPC. MATERIALS AND METHODS: A systematic search strategy was launched trough the main scientific databases including PubMed, Scopus and Cochrane. An additional manual search and a chain citation were performed about potentially relevant papers. The key words used for the search included "eccrine porocarcinoma", "porocarcinoma", "radiotherapy", "radiation therapy", "adjuvant radiotherapy" and "postoperative radiotherapy". RESULTS: A total of 104 publications were identified and 14 papers were included in the final analysis. The only articles found on adjuvant RT in EPC were case reports published between 1996 and 2019. There was a slight female prevalence (57% female/43% male) with a mean age of 65 years (range 37-85). Head-and-neck region was the most frequently involved anatomical site followed by legs. CONCLUSIONS: Adjuvant radiotherapy after surgical removal of EPC could be considered in cases with positive or close margins and in cases with unfavorable histological features. In view of limited literature data and the rarity of EPC the best treatment sequence should always be discussed within the frame of a multidisciplinary setting. ADVANCES IN KNOWLEDGE: adjuvant radiotherapy after surgical removal of EPC could be considered in cases with positive or close margins and in cases with unfavorable histological features.

[Multiple recurrent eccrine porocarcinoma with inguinal metastasis. A case report]. / Porocarcinoma ecrino multirrecidivante con metástasis inguinal. Caso clínico.

BACKGROUND: Eccrine porocarcinoma, first described in 1963, is a rare malignant lesion arising from the eccrine sweat glands. It is usually a primary tumour, or even more common, a malignant degeneration of an eccrine poroma. It usually affects older persons and is located most commonly on the lower extremities. About 20% of eccrine porocarcinoma will recur after treatment. The treatment is wide local excision of the primary lesion. This uncommon skin tumour has a locally aggressive behaviour and a high recurrence rate. CLINICAL CASE: An 82 year-old man presenting with multiple recurrent eccrine porocarcinoma with inguinal metastasis. The treatment was a radical excision and inguinal lymphadenectomy. There were no postoperative complications, but there was local recurrence after six months. CONCLUSION: Early diagnosis and wide excision is the best way to achieve a good prognosis, due to the aggressiveness of this tumour.

Eccrine porocarcinoma with extensive cutaneous metastases.

BACKGROUND: Eccrine porocarcinoma (EPC) is an uncommon malignant neoplasm that originates in the intraepidermal portion of the eccrine sweat duct. Although porocarcinoma is a slow-growing tumor, up to 20% of cases can metastasize to regional lymph nodes, thus increasing mortality. METHODS: We describe the clinical and histopathological features and clinical course of three cases of extensive metastatic EPC diagnosed in our department over the last 10 years. RESULTS: All three patients were women aged 89-96 years. They had numerous skin tumors on the left leg that were histologically and immunohistochemically diagnosed as metastatic EPC. Only one patient had a history of primary porocarcinoma, which had been excised 6 years earlier. The remaining two patients had a previous lesion diagnosed as squamous cell carcinoma. We treated the patients with palliative radiotherapy and/or chemotherapy. Only one patient is currently alive. CONCLUSIONS: The cases of cutaneous and regional metastatic EPC we present occurred in elderly women with major involvement of the left leg. The third case is noteworthy, as the patient presented a long latency period before metastases appeared. Difficulties in the clinical diagnosis--and occasionally histological diagnosis--of primary EPC could delay more aggressive treatment, although optimal treatment does not always guarantee a good prognosis.

Porocarcinoma scalp with high risk features treated with surgery and adjuvant radiotherapy: A case report and review of literature.

Eccrine porocarcinoma is a rare malignant sweat gland tumor arising from the intra dermal part of the gland and accounts for only 0.005% of all epithelial cutaneous tumors. Commonly involved site includes extremities and face. Scalp is a rare site for porocarcinoma with less than 20 reported cases so far. Wide local excision with clear margins remains the treatment of choice. Review of literature revealed a local recurrence rate of 37.5% and a nodal involvement risk of 20%. Porocarcinoma of the scalp is peculiar in that the primary tumor may be large at presentation, making surgery with adequate margins difficult. Adjuvant radiotherapy must be considered in a case to case basis due to the high local recurrence rates compared to other sites of porocarcinoma and should be given to all patients with close margins and extra capsular extension.

Porocarcinoma ecrino: reporte de un caso / Eccrine porocarcinoma: a case report

Los tumores anexiales de la piel representan un grupo heterogéneo de entidades de baja frecuencia de presentación. Su origen es controvertido, quizás a partir de células madres pluripotenciales, aceptándose principalmente dos líneas embriológicas: pilosebáceo-apócrina y ecrina. Se los clasifica en cuatro grupos principales: tumores del folículo piloso, tumores sebáceos, tumores apócrinos y tumores ecrinos. Su distribución anatómica refleja áreas con mayor densidad de anexos cutáneos, presentándose en forma única o múltiple.

Skin adnexal tumors are represented as a heterogeneous group of entities of low frequency range. Its origin is controversial, perhaps from pluripotent stem cells, mainly from two embryological lines: pilosebaceous-apocrine and eccrine. They are classified as four main groups: tumors of the hair follicle, sebaceous tumors, apocrine tumors and eccrine tumors. Their anatomic distribution reflects areas with the highest density of cutaneous adnexal, arising in either single or multiple forms.